The complex patient vad transplant exchange or hospice

1. The Complex Patient:
VAD, Transplant, Exchange or Hospice?
Joseph G. Rogers, MD
Professor of Medicine
Division of Cardiology
Duke University
American College of Cardiology Annual Scientific Sessions
March 14, 2015
San Diego, CA
Disclosures: None
Lee Goldberg, MD
Professor of Medicine
Division of Cardiology
University of Pennsylvania

2. History of Present Illness
76 y/o man presents to ED for evaluation of recurrent cough syncope
• Multiple episodes over previous several months
• Several months of progressive heart failure symptoms
• Orthopnea/PND
• Abdominal distention
• PMH
• LV dysfunction
• Prostate Cancer: Radical Prostatectomy 1998
• HTN
• Gout
• CKD Stage 3: Cr 1.8, GFR 45
• Paroxysmal atrial fibrillation
• LE neuropathy attributed to prior back surgery

3. Meds/Exam
• Carvedilol 3.125 mg bid, allopurinol 200 mg daily, temisartan 80 mg
daily, gabapentin 600 mg at bedtime, warfarin, atorvastatin 10 mg
qHS, furosemide 40 mg daily
• No tobacco, social ETOH, retired executive, widower in relationship
• Fx: CAD, RAD, alzheimer’s
• Afeb-84-131/94
– WD/WN, NAD. Appeared younger than stated age
– Clear lungs
– Irregular rhythm, no murmur or gallop, JVP 4 cm above clavicle at 450
– Bilateral LE edema

4. Laboratory Evaluation
• Na=136, K=3.8, BUN/Cr= 38/1.8.
• INR=2.9, Hct=42%
• CK=339 with MB=10. Troponin T=0.10
• NT-proBNP=7031
• TSH=3.7
• UA: no proteinuria
• Blood type: AB

5. ECG

6. Echo

7. Cardiac Catheterization (after diuresis)
• Normal coronary arteries
• Hemodynamics
– RA=5, PA=35/13, PCWP=14
– CI=1.7
– SVO2=55%

8. Cardiac Biopsy
• Negative SPEP/UPEP
• Negative bone marrow
• No gene analysis performed

9. Cardiac Amyloid
• Three types
– AL – associated with plasma cell dyscrasia (multiple myeloma)
– TTR – mutant transthyretin protein – Familial
• Multiple mutations leading to variable phenotypes
• Val122Ile is associated with predominantly cardiac involvement – 3
to 4% of African Americans
– TTR – wildtype – Senile amyloid – usually cardiac only

10. Genetics Determine Phenotype
Heart Fail Rev (2015) 20:163–178

11. Clinical Syndrome
• Heart failure with preserved ejection fraction
• Heart failure with decreased ejection fraction
– Intolerance to vasodilators
– Intolerance to beta blockers
• Arrhythmias
• Peripheral neuropathy
• Orthostatic hypotension
• AL amyloid can impact many organs
• TTR depending on genotype can impact kidney and other
tissues

12. Prognosis
• Onset of advanced heart failure symptoms
– 6 month survival in AL amyloid
– 43 months in wild-type TTR
– 24 months in familial TTR
• AL amyloid
– Chemotherapy to suppress light chains can lead to some
regression
• TTR amyloid
– No therapy to slow disease
• Drugs now in clinical trial

13. Prognosis
Heart Fail Rev (2015) 20:163–178

14. Treatment
• AL Amyloid
– Treat the underlying plasma cell dyscrasia
– Heart transplant +/- stem cell transplant
• TTR
– Organ transplantation - heart/liver for familial
– Transthyretin stabilizers (diflunisal, tafamidis, AG-1)
– TTR silencers (ALNATTR02, ISIS-TTR(Rx))
– Degraders of amyloid fibrils (doxycycline/TUDCA)

15. Treatment Targets - TTR
Heart Fail Rev (2015) 20:163–178

16. Hospital Course
• Develops more NSVT
• Develops worsening dyspnea, hypotension and renal insufficiency
despite volume
• Started on dopamine

17. Discussion Points
• Should this man be enrolled in a clinical trial for the treatment of
TTR amyloid?
• Should this man be enrolled in hospice?
• Should he receive a DT LVAD?
• Should he receive an extended criteria transplant?
– Age
– Amyloid
– Renal insufficiency

20. Case Conclusion
• Transplanted < 1 month after listing
• Hospital course
– Extubated day of transplant
– To floor POD 1
– Discharged from hospital POD 5
• 2 readmissions
– 24 hours for hydration
– Laminectomy
• Just celebrated 3 year anniversary
– Rows daily
– Cr 1.8
– Normal coronary arteries